Research
Original Investigation
Long-term Management of Adult Vulvar Lichen Sclerosus A Prospective Cohort Study of 507 Women Andrew Lee, MBBS; Jennifer Bradford, MBBS; Gayle Fischer, MD Editorial IMPORTANCE Adult vulvar lichen sclerosis (VLS) may be complicated by loss of vulvar
structure and vulvar carcinoma. There is a lack of evidence as to the ideal method to maintain long-term remission and prevent complications. OBJECTIVES To determine whether long-term preventive topical corticosteroid (TCS) treatment of VLS, with a target outcome of induction and maintenance of normal skin texture and color, reduces the risk of vulvar carcinoma, relieves symptoms, improves function, and preserves vulvar architecture, and to evaluate the adverse effects of treatment. DESIGN, SETTING, AND PARTICIPANTS A prospective longitudinal cohort study was conducted in 507 women with biopsy-proved VLS from January 2, 2008, through September 26, 2014, in the private practice of a dermatologist and a gynecologist in Sydney, Australia. INTERVENTIONS Preventive treatment using TCSs of various potencies, adjusted to meet a
target outcome of normal skin color and texture, with regular long-term follow-up by a dermatologist or gynecologist. MAIN OUTCOMES AND MEASURES Symptoms or signs of VLS, scarring, development of malignant neoplasms, and adverse effects. RESULTS The mean age at presentation was 55.4 years (range, 18-86 years); duration of symptoms at presentation, 5.0 years (range, 0.1-40.0 years); and duration of follow-up, 4.7 years (range, 2.0-6.8 years). Remission was induced with a potent TCS, followed by regular preventive TCS treatment of a potency titrated to achieve the target outcome. Patients were followed up at least annually. A total of 150 patients (29.6%) did not carry out the advised treatment and were considered partially compliant. A total of 357 patients (70.4%) adhered to treatment instructions and were considered compliant. Biopsy-proved squamous cell carcinoma or vulvar intraepithelial neoplasia occurred during follow-up in 0 of the compliant patients vs 7 (4.7%) of the partially compliant patients (P < .001). Suppression of symptoms occurred in 333 (93.3%) compliant patients vs 87 (58.0%) partially compliant patients (P < .001). Adhesions and scarring occurred during follow-up in 12 (3.4%) compliant patients and 60 (40.0%) partially compliant patients (P < .001). Reversible TCS-induced cutaneous atrophy occurred in 4 (1.1%) compliant patients and 3 (2.0%) partially compliant patients. CONCLUSIONS AND RELEVANCE This prospective, single-center, longitudinal cohort study of adult patients with VLS suggests that individualized preventive TCS regimens that achieve objective normality of skin color and texture and are used by compliant patients who attend regular long-term follow-up visits may modify the course of the disease. There was a significant difference in symptom control, scarring, and occurrence of vulvar carcinoma between compliant and partially compliant patients. The adverse effects of TCSs were minimal.
JAMA Dermatol. doi:10.1001/jamadermatol.2015.0643 Published online June 12, 2015.
Author Affiliations: Sydney Medical School Northern, The University of Sydney, New South Wales, Australia (Lee, Fischer); School of Medicine, University of Western Sydney, New South Wales, Australia (Bradford). Corresponding Author: Andrew Lee, MBBS, Sydney Medical School Northern, The University of Sydney, Reserve Rd, St Leonards, New South Wales 2065, Australia ([email protected]).
(Reprinted) E1
Copyright 2015 American Medical Association. All rights reserved.
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Research Original Investigation
Long-term Management of Adult Vulvar Lichen Sclerosus
V
ulvar lichen sclerosus (VLS) is an uncommon skin disease that is frequently complicated by the loss of normal vulvar architecture and less commonly by vulvar squamous neoplasia (VSN), including vulvar intraepithelial neoplasia and invasive squamous cell carcinoma. Before the finding in 19911 that VLS could be suppressed by superpotent topical corticosteroids (TCSs), rates of vulvar squamous cell carcinoma among patients with VLS were reported to be about 5%.2-4 The current accepted management guidelines for VLS advocate the use of superpotent TCSs as first-line treatment to achieve remission. There is good evidence to support this approach.1,5 Less is known about the best practice to maintain remission, the ideal duration of follow-up, and whether long-term management can prevent complications. In a previous study, 2 of us recommended long-term treatment and surveillance for all patients.6 However, other authors have contended that only patients with difficult-to-control VLS should be regularly followed up and have expressed concern that longterm follow-up is a burden on the health care system.7-10 This prospective, single-center cohort study of 507 women with biopsy-proved VLS aims to answer the following questions about long-term management: whether individualized TCS regimens that treat to a target outcome of continued maintenance of normal skin color and texture improve function, achieve preservation of the vulvar architecture, and reduce the risk of VSN without significant adverse effects.
Methods A prospective, single-center cohort study of women with biopsyproved VLS who were treated with TCSs was conducted from January 2, 2008, through September 26, 2014. Patients were considered compliant if they self-reported that they followed treatment instructions “most of the time” or “all of the time” and partially compliant if they self-reported that they followed treatment instructions “some of the time,” “little of the time,” or “none of the time,” either in terms of frequency of application and/or potency of TCS. Our study compared these 2 groups. All patients were evaluated in the private practice of the authors (J.B. and G.F.) in Sydney, Australia. Inclusion criteria were age older than 18 years, biopsy-proved VLS, and having been followed up for a minimum of 2 years. Using previously published studies,2-4 we assumed a 5% risk of VSN. Based on an initial audit of patients attending our practice, only 90 (67.0%) patients were compliant. The other 45 (33.0%) patients were partially compliant. To detect a decrease to 1.0% incidence of VSN in the compliant group compared with the partially compliant group with 80% power at 5% significance, we required a total of 504 patients. At the time of study completion, we had included 357 compliant and 150 partially compliant patients (compliance rate, 70.4%) for a total of 507 patients. This study was approved by the Human Research and Ethics Committee of the Northern Sydney Local Health District. Written or oral consent was obtained from all patients. Data were systematically collected and recorded in a computerized database that was established in 2008 (Genie Solutions). The following characteristics were recorded for all patients: E2
1. Historical features: age, ethnicity, menopausal status (premenopausal, postmenopausal without hormone therapy, or postmenopausal with hormone therapy), duration of symptoms, and previous treatment. 2. Symptoms: itching, vulvar pain, and dyspareunia. 3. Clinical features: distribution (figure of 8, localized on an area of the vulva, clitoris, or perineum, or symmetrical involvement of the vulva), fissuring, telangiectasia, pigmentation, labial fusion (none, anterior, posterior, or both), clitoral hood fusion, and VSN (squamous cell carcinoma, vulvar intraepithelial neoplasia, or none); 73% of patients consented to clinical photographic monitoring. For those who did not consent, detailed clinical descriptions were used. 4. Severity of disease for the purposes of selection of potency of TCSs: defined by degree of hyperkeratosis: mild (1+), moderate (2+), severe (3+), or very severe (4+) (Figure 1). Scarring was not used as a severity marker because it does not change with treatment. 5. Adverse effects: stinging or irritation caused by topical therapy; atrophy evidenced by skin fragility, telangiectasia, and visible veins; and corticosteroid dermatitis evidenced by development of erythema with burning or soreness. Initial treatment regimens were individualized, with the target outcome being an objective return of the vulvar skin to normal color and texture. Patients were initially treated with a single TCS agent, applied daily, to achieve symptom control. The most commonly used agent in 325 (64.1%) patients was betamethasone dipropionate, 0.05%, in optimized vehicle ointment, a superpotent TCS, followed by methylprednisolone aceponate ointment, 0.1%, a midpotency TCS that was used in 156 (30.8%) patients. Clobetasol propionate ointment, 0.05%, an ultrapotent TCS, was used in 17 (3.4%) patients and hydrocortisone ointment, 1%, was used in 9 (1.8%) patients (Table 1). The decision regarding which TCS with which to initiate treatment was made according to the degree of severity of hyperkeratosis (Figure 1): very severe (4+), clobetasol propionate ointment; moderate to severe (2+ or 3+), betamethasone dipropionate ointment; and mild (1+), methylprednisolone aceponate ointment. A small number (9 [1.8%]) of patients had very mild “burnt out” disease and were treated with hydrocortisone ointment. Once disease and symptom suppression had been achieved, long-term preventive management was initiated. A gradual reduction of TCS potency, titrated to the clinical response, was attempted in all patients. Treatment was outcome based, with the target being as close as possible to normal skin color and texture (Figure 2). As long as there were no adverse effects, this treatment was maintained. If atrophy or corticosteroid dermatitis developed, the potency of the TCS was reduced. If hyperkeratosis returned, the potency of the TCS was increased. Patients used the treatment at least 3 times per week. For patients with very severe disease, a potent to superpotent TCS was used daily. Follow-up was conducted every 3 to 6 months for the first 2 years and then at least yearly to ensure that treatment was
JAMA Dermatology Published online June 12, 2015 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archderm.jamanetwork.com/ by a Northern Kentucky University User on 06/16/2015
jamadermatology.com
Long-term Management of Adult Vulvar Lichen Sclerosus
Original Investigation Research
Figure 1. Severity Guide to Treatment of Vulvar Lichen Sclerosus A Mild (1+)
B
C
D Very severe (4+)
Severe (3+)
Moderate (2+)
Grading of hyperkeratosis in vulvar lichen sclerosus used to determine corticosteroid treatment potency.
adequate to maintain the target outcome and to encourage compliance. Patients were not instructed to treat themselves as required, and it was emphasized that treatment should be continued preventively even when asymptomatic. Patients were informed that possible outcomes of poor compliance included cancer and scarring. At follow-up visits, the following features were recorded: self-reported compliance regarding frequency and potency of the TCS used, subjective symptomatic response to treatment, objective clinical response to treatment, maintenance therapy required, progress or onset of adhesions or scarring, adverse effects, and development of squamous cell carcinoma or vulvar intraepithelial neoplasia. At the completion of the 7-year observational period, all data collected from the 507 patients were entered into an Excel 2011 spreadsheet (Microsoft Corporation). Statistical analysis was conducted using SPSS, version 20.0 (SPSS Inc). Descriptive statistics are presented, and Fisher exact tests, Pearson χ 2 , or independent, unpaired, 2-tailed t tests were conducted, as appropriate, to compare outcome rates between those who were compliant and partially compliant. jamadermatology.com
Results Demographic Data The mean age at presentation was 55.4 years (range, 18-86 years), and the mean duration of symptoms before presentation was 5.0 years (range, 0.1-40.0 years). A total of 158 (31.2%) patients were premenopausal, 307 (60.6%) were postmenopausal and not using hormone therapy, and 42 (8.3%) were postmenopausal and using either topical or systemic hormone therapy. The mean duration of follow-up for all patients was 4.7 years (range, 2.0-6.8 years). Most patients (476 [93.9%]) were white. The mean age at presentation of patients who developed VSN was 57.8 years (range, 29-76 years), with a mean duration of symptoms before presentation of 9.4 years (range, 1-30 years).
Clinical Features At presentation, most patients (491 [96.8%]) were symptomatic and most (311 of 414 sexually active patients [75.1%]) experienced dyspareunia. Nearly all women (471 [92.9%]) expe(Reprinted) JAMA Dermatology Published online June 12, 2015
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archderm.jamanetwork.com/ by a Northern Kentucky University User on 06/16/2015
E3
Research Original Investigation
Long-term Management of Adult Vulvar Lichen Sclerosus
Table 1. Suggested Guidelines for Initial Management Degree of Hyperkeratosis 0+ (Burnt out: very mild) 1+ (Mild)
2+ (Moderate)
3+ (Severe)
4+ (Very severe)
Initial Management Mild-potent TCS (in our study, hydrocortisone ointment, 1.0%) Midpotent TCS (in our study, methylprednisolone aceponate ointment, 0.1%) Superpotent TCS (in our study, betamethasone dipropionate, 0.05%, in optimized vehicle ointment) Superpotent TCS (in our study, betamethasone dipropionate, 0.05%, in optimized vehicle ointment) Ultrapotent TCS (in our study, clobetasol propionate ointment, 0.05%)
Frequency Once daily Once daily
Once daily
Twice daily
Twice daily
Abbreviation: TCS, topical corticosteroid.
Figure 2. Suggested Long-term Topical Corticosteroid (TCS) Management of Vulvar Lichen Sclerosus Initial TCS treatment for 6 weeks based on degree of hyperkeratosis Very severe (4+), ultrapotent TCS Moderate to severe (2-3+), superpotent TCS Mild (1+), midpotent TCS Burnt out (0+), mild-potent TCS
Normal skin color and texture
Skin color and texture not normalized
Reduce TCS strength
Continue initial TCS treatment
rienced itching (326 [91.3%] compliant vs 145 [96.7%] partially compliant; P = .02). However, there was a small group (16 [3.2%]) who were asymptomatic, with changes discovered by their primary care physician. In most patients (381 [75.1%]), VLS involved the entire genital area (vulva, perineum, and perianal skin). Structural changes in the vulvar architecture were found in approximately half the patients (262 [51.7%]) at presentation (173 [48.5%] compliant vs 89 [59.3%] partially compliant; P = .03); 82 (16.2%) women had anterior fusion (fusion of clitoral hood or labia minora fused to labia majora), and 58 (11.4%) had posterior fusion resulting in loss of vaginal opening, 122 (24.1%) of whom had both anterior and posterior fusion. Patients who presented with scarring had a mean symptom duration of 5.8 years, and those without scarring had a duration of 4.1 years (P = .006). There was an increased likelihood of scarring with more severe hyperkeratotic disease (1+, 70 [44.8%]; 2+, 88 [46.1%]; and 3-4+, 100 [66.2%]; P
Original Investigation
Long-term Management of Adult Vulvar Lichen Sclerosus A Prospective Cohort Study of 507 Women Andrew Lee, MBBS; Jennifer Bradford, MBBS; Gayle Fischer, MD Editorial IMPORTANCE Adult vulvar lichen sclerosis (VLS) may be complicated by loss of vulvar
structure and vulvar carcinoma. There is a lack of evidence as to the ideal method to maintain long-term remission and prevent complications. OBJECTIVES To determine whether long-term preventive topical corticosteroid (TCS) treatment of VLS, with a target outcome of induction and maintenance of normal skin texture and color, reduces the risk of vulvar carcinoma, relieves symptoms, improves function, and preserves vulvar architecture, and to evaluate the adverse effects of treatment. DESIGN, SETTING, AND PARTICIPANTS A prospective longitudinal cohort study was conducted in 507 women with biopsy-proved VLS from January 2, 2008, through September 26, 2014, in the private practice of a dermatologist and a gynecologist in Sydney, Australia. INTERVENTIONS Preventive treatment using TCSs of various potencies, adjusted to meet a
target outcome of normal skin color and texture, with regular long-term follow-up by a dermatologist or gynecologist. MAIN OUTCOMES AND MEASURES Symptoms or signs of VLS, scarring, development of malignant neoplasms, and adverse effects. RESULTS The mean age at presentation was 55.4 years (range, 18-86 years); duration of symptoms at presentation, 5.0 years (range, 0.1-40.0 years); and duration of follow-up, 4.7 years (range, 2.0-6.8 years). Remission was induced with a potent TCS, followed by regular preventive TCS treatment of a potency titrated to achieve the target outcome. Patients were followed up at least annually. A total of 150 patients (29.6%) did not carry out the advised treatment and were considered partially compliant. A total of 357 patients (70.4%) adhered to treatment instructions and were considered compliant. Biopsy-proved squamous cell carcinoma or vulvar intraepithelial neoplasia occurred during follow-up in 0 of the compliant patients vs 7 (4.7%) of the partially compliant patients (P < .001). Suppression of symptoms occurred in 333 (93.3%) compliant patients vs 87 (58.0%) partially compliant patients (P < .001). Adhesions and scarring occurred during follow-up in 12 (3.4%) compliant patients and 60 (40.0%) partially compliant patients (P < .001). Reversible TCS-induced cutaneous atrophy occurred in 4 (1.1%) compliant patients and 3 (2.0%) partially compliant patients. CONCLUSIONS AND RELEVANCE This prospective, single-center, longitudinal cohort study of adult patients with VLS suggests that individualized preventive TCS regimens that achieve objective normality of skin color and texture and are used by compliant patients who attend regular long-term follow-up visits may modify the course of the disease. There was a significant difference in symptom control, scarring, and occurrence of vulvar carcinoma between compliant and partially compliant patients. The adverse effects of TCSs were minimal.
JAMA Dermatol. doi:10.1001/jamadermatol.2015.0643 Published online June 12, 2015.
Author Affiliations: Sydney Medical School Northern, The University of Sydney, New South Wales, Australia (Lee, Fischer); School of Medicine, University of Western Sydney, New South Wales, Australia (Bradford). Corresponding Author: Andrew Lee, MBBS, Sydney Medical School Northern, The University of Sydney, Reserve Rd, St Leonards, New South Wales 2065, Australia ([email protected]).
(Reprinted) E1
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archderm.jamanetwork.com/ by a Northern Kentucky University User on 06/16/2015
Research Original Investigation
Long-term Management of Adult Vulvar Lichen Sclerosus
V
ulvar lichen sclerosus (VLS) is an uncommon skin disease that is frequently complicated by the loss of normal vulvar architecture and less commonly by vulvar squamous neoplasia (VSN), including vulvar intraepithelial neoplasia and invasive squamous cell carcinoma. Before the finding in 19911 that VLS could be suppressed by superpotent topical corticosteroids (TCSs), rates of vulvar squamous cell carcinoma among patients with VLS were reported to be about 5%.2-4 The current accepted management guidelines for VLS advocate the use of superpotent TCSs as first-line treatment to achieve remission. There is good evidence to support this approach.1,5 Less is known about the best practice to maintain remission, the ideal duration of follow-up, and whether long-term management can prevent complications. In a previous study, 2 of us recommended long-term treatment and surveillance for all patients.6 However, other authors have contended that only patients with difficult-to-control VLS should be regularly followed up and have expressed concern that longterm follow-up is a burden on the health care system.7-10 This prospective, single-center cohort study of 507 women with biopsy-proved VLS aims to answer the following questions about long-term management: whether individualized TCS regimens that treat to a target outcome of continued maintenance of normal skin color and texture improve function, achieve preservation of the vulvar architecture, and reduce the risk of VSN without significant adverse effects.
Methods A prospective, single-center cohort study of women with biopsyproved VLS who were treated with TCSs was conducted from January 2, 2008, through September 26, 2014. Patients were considered compliant if they self-reported that they followed treatment instructions “most of the time” or “all of the time” and partially compliant if they self-reported that they followed treatment instructions “some of the time,” “little of the time,” or “none of the time,” either in terms of frequency of application and/or potency of TCS. Our study compared these 2 groups. All patients were evaluated in the private practice of the authors (J.B. and G.F.) in Sydney, Australia. Inclusion criteria were age older than 18 years, biopsy-proved VLS, and having been followed up for a minimum of 2 years. Using previously published studies,2-4 we assumed a 5% risk of VSN. Based on an initial audit of patients attending our practice, only 90 (67.0%) patients were compliant. The other 45 (33.0%) patients were partially compliant. To detect a decrease to 1.0% incidence of VSN in the compliant group compared with the partially compliant group with 80% power at 5% significance, we required a total of 504 patients. At the time of study completion, we had included 357 compliant and 150 partially compliant patients (compliance rate, 70.4%) for a total of 507 patients. This study was approved by the Human Research and Ethics Committee of the Northern Sydney Local Health District. Written or oral consent was obtained from all patients. Data were systematically collected and recorded in a computerized database that was established in 2008 (Genie Solutions). The following characteristics were recorded for all patients: E2
1. Historical features: age, ethnicity, menopausal status (premenopausal, postmenopausal without hormone therapy, or postmenopausal with hormone therapy), duration of symptoms, and previous treatment. 2. Symptoms: itching, vulvar pain, and dyspareunia. 3. Clinical features: distribution (figure of 8, localized on an area of the vulva, clitoris, or perineum, or symmetrical involvement of the vulva), fissuring, telangiectasia, pigmentation, labial fusion (none, anterior, posterior, or both), clitoral hood fusion, and VSN (squamous cell carcinoma, vulvar intraepithelial neoplasia, or none); 73% of patients consented to clinical photographic monitoring. For those who did not consent, detailed clinical descriptions were used. 4. Severity of disease for the purposes of selection of potency of TCSs: defined by degree of hyperkeratosis: mild (1+), moderate (2+), severe (3+), or very severe (4+) (Figure 1). Scarring was not used as a severity marker because it does not change with treatment. 5. Adverse effects: stinging or irritation caused by topical therapy; atrophy evidenced by skin fragility, telangiectasia, and visible veins; and corticosteroid dermatitis evidenced by development of erythema with burning or soreness. Initial treatment regimens were individualized, with the target outcome being an objective return of the vulvar skin to normal color and texture. Patients were initially treated with a single TCS agent, applied daily, to achieve symptom control. The most commonly used agent in 325 (64.1%) patients was betamethasone dipropionate, 0.05%, in optimized vehicle ointment, a superpotent TCS, followed by methylprednisolone aceponate ointment, 0.1%, a midpotency TCS that was used in 156 (30.8%) patients. Clobetasol propionate ointment, 0.05%, an ultrapotent TCS, was used in 17 (3.4%) patients and hydrocortisone ointment, 1%, was used in 9 (1.8%) patients (Table 1). The decision regarding which TCS with which to initiate treatment was made according to the degree of severity of hyperkeratosis (Figure 1): very severe (4+), clobetasol propionate ointment; moderate to severe (2+ or 3+), betamethasone dipropionate ointment; and mild (1+), methylprednisolone aceponate ointment. A small number (9 [1.8%]) of patients had very mild “burnt out” disease and were treated with hydrocortisone ointment. Once disease and symptom suppression had been achieved, long-term preventive management was initiated. A gradual reduction of TCS potency, titrated to the clinical response, was attempted in all patients. Treatment was outcome based, with the target being as close as possible to normal skin color and texture (Figure 2). As long as there were no adverse effects, this treatment was maintained. If atrophy or corticosteroid dermatitis developed, the potency of the TCS was reduced. If hyperkeratosis returned, the potency of the TCS was increased. Patients used the treatment at least 3 times per week. For patients with very severe disease, a potent to superpotent TCS was used daily. Follow-up was conducted every 3 to 6 months for the first 2 years and then at least yearly to ensure that treatment was
JAMA Dermatology Published online June 12, 2015 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archderm.jamanetwork.com/ by a Northern Kentucky University User on 06/16/2015
jamadermatology.com
Long-term Management of Adult Vulvar Lichen Sclerosus
Original Investigation Research
Figure 1. Severity Guide to Treatment of Vulvar Lichen Sclerosus A Mild (1+)
B
C
D Very severe (4+)
Severe (3+)
Moderate (2+)
Grading of hyperkeratosis in vulvar lichen sclerosus used to determine corticosteroid treatment potency.
adequate to maintain the target outcome and to encourage compliance. Patients were not instructed to treat themselves as required, and it was emphasized that treatment should be continued preventively even when asymptomatic. Patients were informed that possible outcomes of poor compliance included cancer and scarring. At follow-up visits, the following features were recorded: self-reported compliance regarding frequency and potency of the TCS used, subjective symptomatic response to treatment, objective clinical response to treatment, maintenance therapy required, progress or onset of adhesions or scarring, adverse effects, and development of squamous cell carcinoma or vulvar intraepithelial neoplasia. At the completion of the 7-year observational period, all data collected from the 507 patients were entered into an Excel 2011 spreadsheet (Microsoft Corporation). Statistical analysis was conducted using SPSS, version 20.0 (SPSS Inc). Descriptive statistics are presented, and Fisher exact tests, Pearson χ 2 , or independent, unpaired, 2-tailed t tests were conducted, as appropriate, to compare outcome rates between those who were compliant and partially compliant. jamadermatology.com
Results Demographic Data The mean age at presentation was 55.4 years (range, 18-86 years), and the mean duration of symptoms before presentation was 5.0 years (range, 0.1-40.0 years). A total of 158 (31.2%) patients were premenopausal, 307 (60.6%) were postmenopausal and not using hormone therapy, and 42 (8.3%) were postmenopausal and using either topical or systemic hormone therapy. The mean duration of follow-up for all patients was 4.7 years (range, 2.0-6.8 years). Most patients (476 [93.9%]) were white. The mean age at presentation of patients who developed VSN was 57.8 years (range, 29-76 years), with a mean duration of symptoms before presentation of 9.4 years (range, 1-30 years).
Clinical Features At presentation, most patients (491 [96.8%]) were symptomatic and most (311 of 414 sexually active patients [75.1%]) experienced dyspareunia. Nearly all women (471 [92.9%]) expe(Reprinted) JAMA Dermatology Published online June 12, 2015
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archderm.jamanetwork.com/ by a Northern Kentucky University User on 06/16/2015
E3
Research Original Investigation
Long-term Management of Adult Vulvar Lichen Sclerosus
Table 1. Suggested Guidelines for Initial Management Degree of Hyperkeratosis 0+ (Burnt out: very mild) 1+ (Mild)
2+ (Moderate)
3+ (Severe)
4+ (Very severe)
Initial Management Mild-potent TCS (in our study, hydrocortisone ointment, 1.0%) Midpotent TCS (in our study, methylprednisolone aceponate ointment, 0.1%) Superpotent TCS (in our study, betamethasone dipropionate, 0.05%, in optimized vehicle ointment) Superpotent TCS (in our study, betamethasone dipropionate, 0.05%, in optimized vehicle ointment) Ultrapotent TCS (in our study, clobetasol propionate ointment, 0.05%)
Frequency Once daily Once daily
Once daily
Twice daily
Twice daily
Abbreviation: TCS, topical corticosteroid.
Figure 2. Suggested Long-term Topical Corticosteroid (TCS) Management of Vulvar Lichen Sclerosus Initial TCS treatment for 6 weeks based on degree of hyperkeratosis Very severe (4+), ultrapotent TCS Moderate to severe (2-3+), superpotent TCS Mild (1+), midpotent TCS Burnt out (0+), mild-potent TCS
Normal skin color and texture
Skin color and texture not normalized
Reduce TCS strength
Continue initial TCS treatment
rienced itching (326 [91.3%] compliant vs 145 [96.7%] partially compliant; P = .02). However, there was a small group (16 [3.2%]) who were asymptomatic, with changes discovered by their primary care physician. In most patients (381 [75.1%]), VLS involved the entire genital area (vulva, perineum, and perianal skin). Structural changes in the vulvar architecture were found in approximately half the patients (262 [51.7%]) at presentation (173 [48.5%] compliant vs 89 [59.3%] partially compliant; P = .03); 82 (16.2%) women had anterior fusion (fusion of clitoral hood or labia minora fused to labia majora), and 58 (11.4%) had posterior fusion resulting in loss of vaginal opening, 122 (24.1%) of whom had both anterior and posterior fusion. Patients who presented with scarring had a mean symptom duration of 5.8 years, and those without scarring had a duration of 4.1 years (P = .006). There was an increased likelihood of scarring with more severe hyperkeratotic disease (1+, 70 [44.8%]; 2+, 88 [46.1%]; and 3-4+, 100 [66.2%]; P
